title: Endoscopic transnasal decompression for management of basilar invagination in osteogenesis imperfecta.
abstract: Osteogenesis imperfecta (OI) is a disorder of bone development caused by a genetic dysfunction of collagen synthesis. Basilar invagination (BI) is an uncommon but serious complication of OI. Brainstem decompression in OI is undertaken in certain circumstances. Transoral-transpalatopharyngeal ventral decompression with posterior occipitocervical fusion has become the treatment of choice when required. This technical note outlines a novel endoscopic transnasal approach for ventral decompression. The literature is reviewed and a strategy for the management of BI in patients with OI is outlined.
authors.affiliation: Department of Neurosurgery, Westmead Private Hospital, Westmead, Australia.
authors.forename: Mitchell A
authors.initials: MA
authors.lastname: Hansen
authors.forename: Melville J
authors.initials: MJ
authors.lastname: da Cruz
authors.forename: Brian K
authors.initials: BK
authors.lastname: Owler
mesh_terms: D000328:Adult; D019299:Decompression, Surgical / Q000379:methods*; D004724:Endoscopy; D005260:Female; D006801:Humans; D009296:Nasal Cavity; D010013:Osteogenesis Imperfecta / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D010985:Platybasia / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D011859:Radiography